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Classic hairy cell leukemia with an aggressive presentation.

Tyler Fugere1, Susan Harley2, Abhijit Godbole3

  • 1Department of Internal Medicine, University of Arkansas for Medical Sciences , Little Rock , Arkansas.

Proceedings (Baylor University. Medical Center)
|November 4, 2021
PubMed
Summary

Hairy cell leukemia, a rare B cell cancer, can present with severe cytopenias and splenomegaly. Early diagnosis is crucial for effective treatment with purine analogs, leading to excellent patient outcomes.

Keywords:
Cladribinehairy cell leukemialeukocytosisrituximabsplenomegaly

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Hairy cell leukemia (HCL) is a rare, indolent B-cell neoplasm characterized by specific cytologic and immunophenotypic features.
  • Typical presentation involves older adults with pancytopenia and splenomegaly.

Observation:

  • A patient presented with severe anemia, thrombocytopenia, leukocytosis, and splenomegaly.
  • The patient experienced a complicated clinical course including transfusion-refractory pancytopenia, airway compromise due to bleeding, subdural hematoma, and Staphylococcus aureus bacteremia.

Findings:

  • This case underscores the diverse and severe clinical manifestations of HCL.
  • The patient's complex presentation highlights the diagnostic challenges HCL can pose.

Implications:

  • Hairy cell leukemia should be considered in the differential diagnosis for patients presenting with cytopenia and splenomegaly.
  • Prompt diagnosis and treatment with purine analogs are associated with excellent response rates in HCL.